- Email: [email protected]
Clinical significance of cutaneous proteoglycan (mucin) infiltration in patients with mitral valve prolapse
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Many dermatologists routinely use a 30-gauge needle to inject triamcinolone into keloids to minimize pain associated with injections. Although occasionally effective for thin soft keloids, 30-gauge needles are insufficient to deliver adequate medication to firmer lesions. The explanation can be gleamed from Poiseuille’s law, which governs laminar flow4: Pgauge ¼ 8hL ðflow rateÞ=pR4 ; where P is the required gauge pressure propelling corticosteroid solution into a keloid at a particular flow rate, h is the viscosity of the fluid, L is the length of the needle, and R is the inner radius of needle, which is raised to the fourth power. With flow rate held constant, the inner radius of a needle is clearly the most critical factor in determining the required gauge pressure to deliver the fluid. For example, at a given flow rate, the pressure needed with a 30-gauge needle (0.070 mm IR)4 would be approximately 27 times that of using a 23-gauge needle (0.159 mm IR)5 and 576 times that of using a 19-gauge needle (0.343 mm IR)5 of the same length. Before the corticosteroid injection, a slow subcutaneous infiltration of local anesthesia is done to minimize pain. With a 30-gauge needle, an injection of buffered 1% lidocaine with epinephrine (1:100,000) is made either directly under a small keloid (\2 cm) or around a larger lesion. In our experience, the local anesthesia minimizes pain and enhances patient compliance with treatment. In cases where the keloid is so firm that the pressure required for injection exceeds the gauge pressure producible by the human thumb, making multiple longitudinal or cross-sectional passes with a large-bore (at least 23-gauge) 1-in needle through the keloid allows for significant deposition of the corticosteroid within the needle tracts. The solution
RESEARCH Clinical significance of cutaneous proteoglycan (mucin) infiltration in patients with mitral valve prolapse To the Editor: Mitral valve prolapse (MVP) occurs in 2.4% to 5% of the general population1,2 both as a primary (inherited or sporadic) and a secondary condition associated with rheumatic and cardiovascular disease. Most patients with primary MVP are asymptomatic and do not require treatment; rarely, severe complications such as a sudden death2,3 may
is well retained because the entrances of the needle tracts tend to collapse. As a rule, syringes should be equipped with a Luer-lock to prevent disengagement during treatment. In summary, we propose a simple technique to deliver corticosteroid to a firm keloid, utilizing supplies readily available in a dermatology practice and review the physics behind the technique. In our experience, the technique allows an adequate deposition of the corticosteroid, leading to excellent clinical response and patient satisfaction. Gary S. Chuang, MD,a,b Gary S. Rogers, MD,b and Ross Zeltser, MDa,b Departments of Dermatology,a Boston University and Tufts University,b New England Medical Center, Boston, Massachusetts Funding sources: None. Conflicts of interest: None declared. Reprint requests: Ross Zeltser, MD, 185 Kisco Ave, Mount Kisco, NY 10549 E-mail: [email protected] REFERENCES 1. Azad S, Sacks L. Painless steroid injections for hypertrophic scars and keloids. Br J Plast Surg 2002;55:534. 2. Ono N. Pain-free intralesional injection of triamcinolone for the treatment of keloid. Scand J Plast Reconstr Surg Hand Surg 1999;33:89-91. 3. Nduka C, van Dam H, Davis K, Shibu M. Painless steroid injections for hypertrophic scars and keloids. Br J Plast Surg 2003;56:842. 4. Pfitzner J. Poiseuille and his law. Anaesthesia 1976;31:273-5. 5. Sigma Aldrich Website. Syringe needle conversion chart. Available at: http://www.sigmaaldrich.com/Area_of_Interest/ Research_Essentials/Chemicals/Key_Resources/Technical_Library/ Needle_Gauge_Chart.html. Accessed March 25, 2006. doi:10.1016/j.jaad.2008.02.017
LETTERS occur. Sudden death in these cases has been associated with myxomatous degeneration and subsequent disruption of the mitral valve leaflets.1 Predicting patients at risk may be beneficial and we hypothesized that myxomatous degeneration of mitral valves may be seen in context with myxomatous changes of the skin. We, therefore, assessed proteoglycan content of clinically normal-appearing skin in patients with primary MVP. Eight patients (5 men) with a mean age of 44.5 years (range 33-52) and primary MVP were
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compared with 6 (4 men) age-matched healthy control subjects. Patients and control subjects each had two 4-mm punch biopsies performed from forearm. One specimen was stained with hematoxylin-eosin and colloidal iron to determine the presence of proteoglycans (read by two masked dermatopathologists). Light microscopic changes in proteoglycans infiltration were graded in a 4step scale.4 The second specimen was snap frozen (e708C) and tested for hexosamine content by using a dimethylmethylene blue dye-binding technique to quantify proteoglycan content.5 Results were compared using the Student t test. Proteoglycans were increased in all patients with MVP compared with control subjects. Semiquantitative analysis of proteoglycan deposition by hematoxylin-eosin and colloidal iron staining was 3.4 in patients with MVP compared with 1.0 in control subjects. Quantitative proteoglycan analysis in patients with MVP was significantly greater (0.629 mg/g) compared with control subjects (0.4 mg/g) (P ¼ .006). Changes in the amount and type of proteoglycans has been reported in a variety of dermatologic conditions associated with MVP such as in Marfan syndrome, Ehlers-Danlos syndrome, cutis laxa, and other heritable disorders of connective tissue.6 Moreover, in a study of 24 patients’ hearts with severe myxoid transformation of the mitral valve, 18 of whom died suddenly, an increased accumulation of proteoglycans was found in the conduction system suggesting the local derangement of valvular tissue in MVP, reflecting a more general myxomatous alteration of the connective tissue.7 Our study, which requires validation, suggests skin analysis could be a simple method to evaluate a correlation between MVP and myxomatous changes in the skin. If, in fact, a high content of proteoglycan in the skin is associated with severe myxomatous alteration of the mitral valve structure as we suggest, these patients might be candidates for further study of their cardiac conduction system to identify those at greater risk for subsequent development of complex arrhythmias and sudden death. The authors thank George W. Elgart, MD, and Robert S. Kirsner, MD, for their mentorship for the project.
Paolo Romanelli, MD,a Renzo Romanelli, MD,y Franco Rongioletti, MD,b Marco Romanelli, MD, PhD,c Jennifer Trent, MD,a Carlos Ricotti, MD,a Indushekhar Persaud, BS,a J. C. Pastor, MD,d and Eduardo de Marchena, MDd Departments of Dermatology and Cutaneous Surgery,a and Cardiology,d L. Miller School of Medicine, Miami, Florida; Department of Dermatology,
University of Genoa, Italyb; Department of Dermatology, University of Pisa, Italyc yDeceased Funding sources: None. Conflicts of interest: None declared. Reprint requests: Franco Rongioletti, MD, DISEM, Section of Dermatology, University of Genoa Viale Benedetto XV, 7 16132 Genoa, Italy E-mail: [email protected] REFERENCES 1. Nishimura RA, McGoon MD, Shub C, Miller FA, Ilstrup DM, Tajik J. Echocardiographically documented mitral-valve prolapse: longterm follow-up of 237 patients. N Engl J Med 1985;313:1305-9. 2. Freed LA, Levy D, Levine RA, Larson MG, Evans JC, Fuller DC, et al. Prevalence and clinical outcome of mitral valve prolapse. N Engl J Med 1999;341:1-7. 3. Zuppirolo A, Rinaldi M, Kramer-Fox R, Favilli S, Roman MJ, Devereux RB. Natural history of mitral valve prolapse. Am J Cardiol 1995;75:1028-33. 4. King BD, Clark MA, Baba N, Kilman JW, Wooley CF. ‘‘Myxomatous’’ mitral valves: collagen dissolution as the primary defect. Circulation 1982;66:288-96. 5. Maroudas A, Thomas H. A simple physicochemical micromethod for determining fixed anionic groups in connective tissue. Biochim Biophys Acta 1970;215:214-6. 6. Glesby MJ, Pyeritz RE. Association of mitral valve prolapse and systemic abnormalities of connective tissue: a phenotypic continuum. JAMA 1989;262:523-8. 7. Morales AR, Romanelli R, Boucek RJ, Tate LG, Alvarez RT, Davis JT. Myxoid heart disease: an assessment of extravalvular cardiac pathology in severe mitral valve prolapse. Hum Pathol 1992; 23:129-37. doi:10.1016/j.jaad.2008.03.039
Medallion-like dermal dendrocyte hamartoma in a 36-year-old male To the Editor: Medallion-like dermal dendrocyte hamartoma (MLDDH) is a recently described, congenital cutaneous lesion, with only five cases reported in the literature, all in otherwise healthy females.1-3 The clinical and histologic features are remarkably similar in all patients. Clinically, it presents at birth as a solitary, well circumscribed, asymptomatic, red-brown, atrophic, medallion-shaped patch on the upper trunk or neck. The surface is slightly wrinkled and underlying blood vessels may be visible. Histologically, it is characterized by epidermal atrophy and a dermal proliferation of spindle cells, which stain positively for factor XIIIa and/or CD34, consistent with dermal dendrocytes. We report the first case of this rare lesion in an adult male. A 36-year-old Latino male presented to our dermatology clinic with a 16-cm, dumbbell-shaped, erythematous, atrophic, pliable patch on the mid-chest
J AM ACAD DERMATOL JULY 2008
Many dermatologists routinely use a 30-gauge needle to inject triamcinolone into keloids to minimize pain associated with injections. Although occasionally effective for thin soft keloids, 30-gauge needles are insufficient to deliver adequate medication to firmer lesions. The explanation can be gleamed from Poiseuille’s law, which governs laminar flow4: Pgauge ¼ 8hL ðflow rateÞ=pR4 ; where P is the required gauge pressure propelling corticosteroid solution into a keloid at a particular flow rate, h is the viscosity of the fluid, L is the length of the needle, and R is the inner radius of needle, which is raised to the fourth power. With flow rate held constant, the inner radius of a needle is clearly the most critical factor in determining the required gauge pressure to deliver the fluid. For example, at a given flow rate, the pressure needed with a 30-gauge needle (0.070 mm IR)4 would be approximately 27 times that of using a 23-gauge needle (0.159 mm IR)5 and 576 times that of using a 19-gauge needle (0.343 mm IR)5 of the same length. Before the corticosteroid injection, a slow subcutaneous infiltration of local anesthesia is done to minimize pain. With a 30-gauge needle, an injection of buffered 1% lidocaine with epinephrine (1:100,000) is made either directly under a small keloid (\2 cm) or around a larger lesion. In our experience, the local anesthesia minimizes pain and enhances patient compliance with treatment. In cases where the keloid is so firm that the pressure required for injection exceeds the gauge pressure producible by the human thumb, making multiple longitudinal or cross-sectional passes with a large-bore (at least 23-gauge) 1-in needle through the keloid allows for significant deposition of the corticosteroid within the needle tracts. The solution
RESEARCH Clinical significance of cutaneous proteoglycan (mucin) infiltration in patients with mitral valve prolapse To the Editor: Mitral valve prolapse (MVP) occurs in 2.4% to 5% of the general population1,2 both as a primary (inherited or sporadic) and a secondary condition associated with rheumatic and cardiovascular disease. Most patients with primary MVP are asymptomatic and do not require treatment; rarely, severe complications such as a sudden death2,3 may
is well retained because the entrances of the needle tracts tend to collapse. As a rule, syringes should be equipped with a Luer-lock to prevent disengagement during treatment. In summary, we propose a simple technique to deliver corticosteroid to a firm keloid, utilizing supplies readily available in a dermatology practice and review the physics behind the technique. In our experience, the technique allows an adequate deposition of the corticosteroid, leading to excellent clinical response and patient satisfaction. Gary S. Chuang, MD,a,b Gary S. Rogers, MD,b and Ross Zeltser, MDa,b Departments of Dermatology,a Boston University and Tufts University,b New England Medical Center, Boston, Massachusetts Funding sources: None. Conflicts of interest: None declared. Reprint requests: Ross Zeltser, MD, 185 Kisco Ave, Mount Kisco, NY 10549 E-mail: [email protected] REFERENCES 1. Azad S, Sacks L. Painless steroid injections for hypertrophic scars and keloids. Br J Plast Surg 2002;55:534. 2. Ono N. Pain-free intralesional injection of triamcinolone for the treatment of keloid. Scand J Plast Reconstr Surg Hand Surg 1999;33:89-91. 3. Nduka C, van Dam H, Davis K, Shibu M. Painless steroid injections for hypertrophic scars and keloids. Br J Plast Surg 2003;56:842. 4. Pfitzner J. Poiseuille and his law. Anaesthesia 1976;31:273-5. 5. Sigma Aldrich Website. Syringe needle conversion chart. Available at: http://www.sigmaaldrich.com/Area_of_Interest/ Research_Essentials/Chemicals/Key_Resources/Technical_Library/ Needle_Gauge_Chart.html. Accessed March 25, 2006. doi:10.1016/j.jaad.2008.02.017
LETTERS occur. Sudden death in these cases has been associated with myxomatous degeneration and subsequent disruption of the mitral valve leaflets.1 Predicting patients at risk may be beneficial and we hypothesized that myxomatous degeneration of mitral valves may be seen in context with myxomatous changes of the skin. We, therefore, assessed proteoglycan content of clinically normal-appearing skin in patients with primary MVP. Eight patients (5 men) with a mean age of 44.5 years (range 33-52) and primary MVP were
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J AM ACAD DERMATOL VOLUME 59, NUMBER 1
compared with 6 (4 men) age-matched healthy control subjects. Patients and control subjects each had two 4-mm punch biopsies performed from forearm. One specimen was stained with hematoxylin-eosin and colloidal iron to determine the presence of proteoglycans (read by two masked dermatopathologists). Light microscopic changes in proteoglycans infiltration were graded in a 4step scale.4 The second specimen was snap frozen (e708C) and tested for hexosamine content by using a dimethylmethylene blue dye-binding technique to quantify proteoglycan content.5 Results were compared using the Student t test. Proteoglycans were increased in all patients with MVP compared with control subjects. Semiquantitative analysis of proteoglycan deposition by hematoxylin-eosin and colloidal iron staining was 3.4 in patients with MVP compared with 1.0 in control subjects. Quantitative proteoglycan analysis in patients with MVP was significantly greater (0.629 mg/g) compared with control subjects (0.4 mg/g) (P ¼ .006). Changes in the amount and type of proteoglycans has been reported in a variety of dermatologic conditions associated with MVP such as in Marfan syndrome, Ehlers-Danlos syndrome, cutis laxa, and other heritable disorders of connective tissue.6 Moreover, in a study of 24 patients’ hearts with severe myxoid transformation of the mitral valve, 18 of whom died suddenly, an increased accumulation of proteoglycans was found in the conduction system suggesting the local derangement of valvular tissue in MVP, reflecting a more general myxomatous alteration of the connective tissue.7 Our study, which requires validation, suggests skin analysis could be a simple method to evaluate a correlation between MVP and myxomatous changes in the skin. If, in fact, a high content of proteoglycan in the skin is associated with severe myxomatous alteration of the mitral valve structure as we suggest, these patients might be candidates for further study of their cardiac conduction system to identify those at greater risk for subsequent development of complex arrhythmias and sudden death. The authors thank George W. Elgart, MD, and Robert S. Kirsner, MD, for their mentorship for the project.
Paolo Romanelli, MD,a Renzo Romanelli, MD,y Franco Rongioletti, MD,b Marco Romanelli, MD, PhD,c Jennifer Trent, MD,a Carlos Ricotti, MD,a Indushekhar Persaud, BS,a J. C. Pastor, MD,d and Eduardo de Marchena, MDd Departments of Dermatology and Cutaneous Surgery,a and Cardiology,d L. Miller School of Medicine, Miami, Florida; Department of Dermatology,
University of Genoa, Italyb; Department of Dermatology, University of Pisa, Italyc yDeceased Funding sources: None. Conflicts of interest: None declared. Reprint requests: Franco Rongioletti, MD, DISEM, Section of Dermatology, University of Genoa Viale Benedetto XV, 7 16132 Genoa, Italy E-mail: [email protected] REFERENCES 1. Nishimura RA, McGoon MD, Shub C, Miller FA, Ilstrup DM, Tajik J. Echocardiographically documented mitral-valve prolapse: longterm follow-up of 237 patients. N Engl J Med 1985;313:1305-9. 2. Freed LA, Levy D, Levine RA, Larson MG, Evans JC, Fuller DC, et al. Prevalence and clinical outcome of mitral valve prolapse. N Engl J Med 1999;341:1-7. 3. Zuppirolo A, Rinaldi M, Kramer-Fox R, Favilli S, Roman MJ, Devereux RB. Natural history of mitral valve prolapse. Am J Cardiol 1995;75:1028-33. 4. King BD, Clark MA, Baba N, Kilman JW, Wooley CF. ‘‘Myxomatous’’ mitral valves: collagen dissolution as the primary defect. Circulation 1982;66:288-96. 5. Maroudas A, Thomas H. A simple physicochemical micromethod for determining fixed anionic groups in connective tissue. Biochim Biophys Acta 1970;215:214-6. 6. Glesby MJ, Pyeritz RE. Association of mitral valve prolapse and systemic abnormalities of connective tissue: a phenotypic continuum. JAMA 1989;262:523-8. 7. Morales AR, Romanelli R, Boucek RJ, Tate LG, Alvarez RT, Davis JT. Myxoid heart disease: an assessment of extravalvular cardiac pathology in severe mitral valve prolapse. Hum Pathol 1992; 23:129-37. doi:10.1016/j.jaad.2008.03.039
Medallion-like dermal dendrocyte hamartoma in a 36-year-old male To the Editor: Medallion-like dermal dendrocyte hamartoma (MLDDH) is a recently described, congenital cutaneous lesion, with only five cases reported in the literature, all in otherwise healthy females.1-3 The clinical and histologic features are remarkably similar in all patients. Clinically, it presents at birth as a solitary, well circumscribed, asymptomatic, red-brown, atrophic, medallion-shaped patch on the upper trunk or neck. The surface is slightly wrinkled and underlying blood vessels may be visible. Histologically, it is characterized by epidermal atrophy and a dermal proliferation of spindle cells, which stain positively for factor XIIIa and/or CD34, consistent with dermal dendrocytes. We report the first case of this rare lesion in an adult male. A 36-year-old Latino male presented to our dermatology clinic with a 16-cm, dumbbell-shaped, erythematous, atrophic, pliable patch on the mid-chest